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Published on: November 17, 2021
Outcomes of multidisciplinary management in pediatric low-grade gliomas
Kevin S Oh1, Jonathan Hung, Patricia L Robertson
1Department of Radiation Oncology, University of Michigan Health System, Ann Arbor, MI, USA. koh2@partners.org
Insights
Pediatric low-grade gliomas (LGGs) in children under 5 years old, especially those in the optic pathway/hypothalamus, have a higher risk of progression but not worse survival. Adjuvant radiation therapy (RT) improves progression-free survival (FFP) but not overall survival (OS) when gross total resection is not achieved.
Area of Science:
- Pediatric neuro-oncology
- Neurosurgery
- Radiation oncology
Background:
- Low-grade gliomas (LGGs) are the most common brain tumors in children.
- Multidisciplinary management is crucial for optimizing outcomes in pediatric LGGs.
- Understanding prognostic factors is essential for tailoring treatment strategies.
Purpose of the Study:
- To evaluate treatment outcomes for pediatric low-grade gliomas (LGGs) managed in a multidisciplinary setting.
- To identify prognostic factors influencing progression-free survival (FFP) and overall survival (OS).
- To assess the role of radiation therapy (RT) in managing pediatric LGGs.
Main Methods:
- Retrospective study of 181 pediatric patients with Grade I-II gliomas.
- Analysis of freedom from progression (FFP) and overall survival (OS) using log-rank and Cox proportional hazards models.
- Separate evaluation for patients with and without neurofibromatosis Type 1 (NF1).
Main Results:
- In non-NF1 patients, 7-year FFP was 67% and OS was 94%.
- Tumor location (optic pathway/hypothalamus) and age ≤5 years were associated with worse FFP.
- Gross total resection (GTR) improved FFP and OS; adjuvant RT improved FFP but not OS in subtotal resection cases.
Conclusions:
- Pediatric LGGs in young children (≤5 years) with optic pathway/hypothalamus tumors are prone to progression, but salvage therapy ensures good overall survival.
- Adjuvant RT can be reserved as salvage therapy, as it improves FFP but not OS when GTR is not achieved.
- Multidisciplinary management and careful consideration of prognostic factors are key in treating pediatric LGGs.
Purpose:
To evaluate the outcomes in pediatric low-grade gliomas managed in a multidisciplinary setting.
Methods And Materials:
We conducted a single-institution retrospective study of 181 children with Grade I-II gliomas. Log-rank and stepwise Cox proportional hazards models were used to analyze freedom from progression (FFP) and overall survival (OS).
Results:
Median follow-up was 6.4 years. Thirty-four (19%) of patients had neurofibromatosis Type 1 (NF1) and because of their favorable prognosis were evaluated separately. In the 147 (81%) of patients without NF1, actuarial 7-year FFP and OS were 67 ± 4% (standard error) and 94 ± 2%, respectively. In this population, tumor location in the optic pathway/hypothalamus was associated with worse FFP (39% vs. 76%, p < 0.0003), but there was no difference in OS. Age ≤5 years was associated with worse FFP (52% vs. 75%, p < 0.02) but improved OS (97% vs. 92%, p < 0.05). In those with tissue diagnosis, gross total resection (GTR) was associated with improved 7-year FFP (81% vs. 56%, p < 0.02) and OS (100% vs. 90%, p < 0.03). In a multivariate model, only location in the optic pathway/hypothalamus predicted worse FFP (p < 0.01). Fifty patients received radiation therapy (RT). For those with less than GTR, adjuvant RT improved FFP (89% vs. 49%, p < 0.003) but not OS. There was no difference in OS between patient groups given RT as adjuvant vs. salvage therapy. In NF1 patients, 94% of tumors were located in the optic pathway/hypothalamus. With a conservative treatment strategy in this population, actuarial 7-year FFP and OS were 73 ± 9% and 100%, respectively.
Conclusions:
Low-grade gliomas in children ≤5 years old with tumors in the optic pathway/hypothalamus are more likely to progress, but this does not confer worse OS because of the success of salvage therapy. When GTR is not achieved, adjuvant RT improves FFP but not OS. Routine adjuvant RT can be avoided and instead reserved as salvage.
